Articles: amyloidosis.
-
Multiple nodules of AA (reactive systemic) amyloid were identified at necropsy in the lungs of a patient with Crohn's disease. No other organs were involved. Nodular pulmonary amyloidosis is usually caused by deposition of AL (primary) amyloid.
-
Am. J. Clin. Pathol. · Mar 1993
Case ReportsLocalized pleural microdeposition of type A amyloid in a patient with rheumatoid pleuritis. Histologic distinction from pleural involvement in systemic amyloidosis.
Decorticated pleural tissue from a 74-year-old man with rheumatoid arthritis, an exudative pleural effusion, and normal left ventricular function contained microscopic deposits of amyloid A protein, localized to the interface between a deep layer of dense fibrocollagen and a layer of granulation tissue beneath a surface fibrin exudate. Previously reported instances of pleural amyloid deposition have occurred in patients with presumed systemic amyloidosis, and most such effusions are the result of congestive heart failure. This patient had no evidence of systemic amyloidosis, and this case appeared to be an example of an unusual form of localized amyloidosis, so-called periinflammatory amyloidosis A. These observations suggest that amyloid in a pleural biopsy should not be construed per se to be diagnostic of systemic amyloidosis.
-
J Am Soc Echocardiogr · Jan 1993
Case ReportsDouble cardiomyopathy: coexistent cardiac amyloidosis and hypertrophic obstructive cardiomyopathy.
A combination of hypertrophic obstructive cardiomyopathy (HOCM) and cardiac amyloidosis in the same patient is very rare. Clinical diagnosis could be extremely difficult and may require myocardial biopsy. ⋯ Only after the patient expired from severe, intractable heart failure did the autopsy findings confirm the association of HOCM. We believe that the combination of the two cardiomyopathic processes is very rare and makes treatment extremely difficult.
-
A retrospective study of 79 juvenile arthritic patients with reactive amyloidosis for a mean of 10 years (3 months-24.25 years) from the onset of amyloidosis was performed. Eighty percent of those treated with chlorambucil (n = 57) were alive compared with 23.5% of patients not treated with chlorambucil (n = 19) 10 years after diagnosis. Renal failure was the cause of death in 82.3% and infection in 11.7%. ⋯ Analysis of their fertility status showed that there were 5 normal births in 3 women and 2 terminations of pregnancy in 23 chlorambucil-treated women of child bearing age. Six women had ovarian failure. None of the male patients fathered a child.
-
Doppler echocardiography is useful in assessing diastolic dysfunction. Pulsed-wave Doppler echocardiographic interrogation of the atrioventricular valves and the central veins has been used in conjunction with respiratory monitoring to characterize abnormal diastolic function of the heart in diseases such as constrictive pericarditis, restrictive cardiomyopathy, and cardiac tamponade. ⋯ Cardiac amyloidosis shows a spectrum of the Doppler flows which evolve as the disease progresses from abnormal relaxation in the early phase to pseudo-normal in the intermediate phase and to restrictive in the advanced phase. Doppler echocardiography can be used to assess the hemodynamic significance of pericardial effusion and to detect cardiac tamponade.